B0589 - Painless Jaundice With a Significantly Elevated CA 19-9: It Is Not Always Pancreaticobiliary Cancer

David Jonason, MD¹, Aisha Mohamed, MS², Nicha Wongjarupong, MD³, Oyedele Adeyi, MD¹, Jeffrey H. Albrecht, MD¹, Guru Trikudanathan, MD^1
1University of Minnesota Medical Center, Minneapolis, MN; ²University of Minnesota Medical School, Minneapolis, MN; ³University of Minnesota Medical Center, Hennepin County Medical Center, Minneapolis, MN

Introduction: Carbohydrate antigen 19-9 (CA 19-9) is often very high in pancreaticobiliary cancers but can be substantially elevated in benign diseases too. We present a case of painless jaundice with a significantly elevated CA 19-9 from vanishing bile duct syndrome (VBDS).

Case Description/Methods: 65-year-old white male with history of obesity, hypertension, hyperlipidemia, prior cholecystectomy and no family history of pancreatic cancer presented with two weeks of painless jaundice and 10 pounds unintentional weight loss. Physical exam noted scleral icterus and jaundice with a T.Bili of 23 mg/dL (Table 1). CT scan showed intrahepatic bile duct dilation and a 10 mm common bile duct without a discrete mass. CA 19-9 was elevated to 1,480 U/mL, concerning for malignancy. ERCP found a low-grade main bile duct stricture which was stented. Bile duct brushings and fine needle aspiration of the pancreas were negative for malignancy. Biliary dilation was resolved on follow-up MRCP but his bilirubin remained high. Infectious and autoimmune workup was unremarkable. Repeat cholangiography and brushings confirmed no obstruction or malignancy (Fig.1a). An infiltrative versus drug induced liver injury was suspected. EUS-guided liver biopsies revealed cholestasis with dilated bile canaliculi, hepatocytes with ballooning degeneration and a paucity of bile ductules. Cytokeratin-7 immunostaining demonstrated > 50% ductopenia, confirming VBDS (Fig.1b,c). Follow up at hepatology clinic determined triamterene-HCTZ, atorvastatin or omeprazole stopped during hospitalization to be the likely culprit. His CA 19-9, LFTs and symptoms improved over the following weeks (Table 1).

Discussion: VBDS is an acquired cholestatic liver disease that may mimic the presentation of pancreatic cancer. CA 19-9 can be substantially elevated through T-cell mediated destruction of small bile ducts causing impaired excretion, inflammatory production, and decreased clearance. Severity of liver damage depends on the duration of injury and degree of bile duct loss at the time of diagnosis. Prompt diagnosis is important to prevent cirrhotic progression, improve outcomes, and avoid unnecessary treatments. Recognizing its association with an elevated CA 19-9 may help with this, but is scarcely reported in the literature. CA 19-9 is not typically checked in cases of VBDS but is likely commonly elevated. Providers should be aware of this association when faced with a patient with painless jaundice and a significantly elevated CA 19-9 but no obvious malignancy.




Disclosures:


David Jonason, MD¹, Aisha Mohamed, MS², Nicha Wongjarupong, MD³, Oyedele Adeyi, MD¹, Jeffrey H. Albrecht, MD¹, Guru Trikudanathan, MD¹. B0589 - Painless Jaundice With a Significantly Elevated CA 19-9: It Is Not Always Pancreaticobiliary Cancer, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.